Primary sclerosing cholangitis is a chronic inflammatory disease described by cholestasis and progressive stricturing and destruction of the intrahepatic and extrahepatic biliary tree. There is growing evidence suggesting the involvement of multiple genetic, environmental, microbiological and particularly autoimmune factors contributing to disease development. Primary sclerosing cholangitis is described by the interplay of inflammation, fibrosis, and cholestasis. A cholestatic picture of liver function with an elevation in serum alkaline phosphatase level is the biochemical hallmark of primary sclerosing cholangitis, although certain patients perhaps have normal alkaline phosphatase levels. Effective medical treatment of primary sclerosing cholangitis has been inhibited by uncertainty regarding the pathogenesis of the disease and the factors responsible for its progression. Ursodeoxycholic acid is a hydrophilic bile acid which, in moderate doses, is thought to exert its mechanisms of action chiefly through protective effects on cholangiocytes by decreasing hydrophobicity and toxicity of bile via the stimulation of hepatobiliary synthesis, and a direct effect on adaptive immunity e.g. by preventing dendritic cells.
Pathophysiology, Diagnosis and Management of Primary Sclerosing Cholangitis